摘要目的:初步探索法瓦病（fibroadipose vascular anomaly ，FAVA）的诊疗原则。方法:收集西安国际医学中心医院2019年10月1日至2020年10月1日收治的FAVA患儿8例的临床特征、影像学、治疗过程及随访情况等资料。其中，男2例，女6例；年龄为（11. 8±3. 0）岁，范围为9~17岁；病程为（5. 9±3. 4）年，范围为1~12年。1例患儿就诊时肘关节已骨性融合，口服西罗莫司随访，7例患儿进行以外科手术为基础的综合治疗。结果:患儿就诊时均有持续性疼痛，部分患儿出现肌肉挛缩或关节畸形。2例仅有持续性疼痛的患儿，予以根治性切除，术后3个月随访时患儿疼痛消失。1例伴有肌肉挛缩的患儿，予以根治性切除后行康复训练，术后3个月随访时，疼痛完全消失，踝关节背曲无明显受限。2例关节畸形患儿，予以根治性切除及关节松解，术后疼痛消失，关节畸形得到矫正。另2例关节畸形患儿行分期根治性切除，术后口服西罗莫司，术后2个月随访时疼痛已明显缓解，按计划于4个月后行二期手术。1例口服西罗莫司随访疼痛明显缓解。结论:FAVA的临床症状及影像学表现虽然有特征性但由于病例较罕见，所以容易误诊，因此FAVA的早诊断、早治疗至关重要。以外科手术为基础的综合治疗对FAVA是有效的，关键在于掌握手术原则。

abstractsObjective:To explore the diagnosis and treatment principles of fibroadipose vascular anomaly (FAVA).Methods:A total of 8 hospitalized FAVA children were reviewed from October 1, 2019 to October 1, 2020. Clinical characteristics, imaging data, treatment outcomes and follow-up results were analyzed. There were 2 boys and 6 girls with an average age of (11. 8±3. 0) (9-17) years and an average disease course of (5. 9±3. 4) (1-12) years. One child had bone fusion of elbow joint at the time of admission. After oral sirolimus, pain was significantly relieved. The remainders received comprehensive treatments based upon surgical operation.Results:All of them had persistent pain and some developed muscle contracture or joint deformity. Radical resection was performed in 2 children with only persistent pain and pain disappeared completely during a follow-up period of 3 months. After radical resection, rehabilitation training was offered for 1 child with muscle contracture. At Month 3 post-operation, pain disappeared completely and there was no obvious limitation of ankle dorsalis. In 2 cases of joint deformity undergoing radical resection and joint release, postoperative pain disappeared and joint deformity was significantly corrected. Another 2 children with joint malformations underwent staged radical resection. After oral sirolimus, pain became significantly alleviated within two months and reoperation was scheduled for 4 months later.Conclusion:Clinical and radiographic manifestations of FAVA are generally characteristic. However, FAVA is rare and easily misdiagnosed. Early diagnosis and early treatment are critical. Comprehensive treatment based upon surgical operation is effective. And understanding its surgical principles is essential.